purpura

[per´pu-rah]

a hemorrhagic disease characterized by extravasation of blood into the tissues, under the skin, and through the mucous membranes, and producing spontaneous bruises, ecchymoses, and petechiae (small hemorrhagic spots) on the skin. (See plate in Dermatology Atlas.) When accompanied by a decrease in the circulating platelets, it is called thrombocytopenic purpura; when there is no decrease in the platelet count, it is called nonthrombocytopenic purpura. adj., adj purpu´ric.

There are two general types of thrombocytopenic purpura: primary or idiopathic, in which the cause is unknown, and secondary or symptomatic, which may be associated with exposure to drugs or other chemical agents, systemic diseases such as multiple myeloma and leukemia, diseases affecting the bone marrow or spleen, and infectious diseases such as rubella (German measles).

Symptoms. The outward manifestations and laboratory findings of primary and secondary thrombocytopenic purpura are similar. There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. The platelet count is below 100,000 per cubic millimeter of blood and may go as low as 10,000 per cubic millimeter (normal count is about 250,000 per cubic millimeter). The bleeding time is prolonged and clot retraction is poor. Coagulation time is normal.

Treatment. Differential diagnosis is necessary to determine the type of purpura present and to eliminate the cause if it can be determined. General measures include protection of the patient from trauma, elective surgery, and tooth extractions, any one of which may lead to severe or even fatal hemorrhage. In the thrombocytopenic form, corticosteroids may be administered when the purpura is moderately severe and of short duration. Splenectomy is indicated when other, more conservative measures fail and is successful in a majority of cases. In some instances, especially in children, there may be spontaneous and permanent recovery from idiopathic purpura. (See Atlas 2, Part Q.)

annular telangiectatic purpura a rare form in which pinpoint reddened lesions coalesce to form a ring-shaped or continuously linked pattern, commonly beginning in the lower extremities and becoming generalized. Called also Majocchi's disease.

purpura ful´minans a form of nonthrombocytopenic purpura seen mainly in children, usually after an infectious disease, marked by fever, shock, anemia, and sudden, rapidly spreading symmetrical skin hemorrhages of the lower limbs, often associated with extensive intravascular thromboses and gangrene.

idiopathic thrombocytopenic purpurathrombocytopenic purpura not directly associated with any definable systemic disease, although it often follows a systemic infection; the cause is thought to be an IgG immunoglobulin that acts as an antibody against platelets, causing ecchymoses, petechiae, and other bleeding. There are both acute and chronic forms: the acute form has a sudden onset, is more common in children, and usually resolves spontaneously within a few months; the chronic form has a slower onset, is more common in adults, and may be recurrent.

nonthrombocytopenic purpura purpura without any decrease in the platelet count of the blood. In such cases the cause is either abnormal capillary fragility or a clotting factor deficiency.

id·i·o·path·ic throm·bo·cy·to·pe·nic pur·pu·ra (ITP),

a systemic illness characterized by extensive ecchymoses and hemorrhages from mucous membranes and very low platelet counts; resulting from platelet destruction by macrophages due to an antiplatelet factor; childhood cases are usually brief and rarely present with intracranial hemorrhages, but adult cases are often recurrent and have a higher incidence of grave bleeding, especially intracranial.

thrombocytopenic purpura

a bleeding disorder characterized by a marked decrease in the number of platelets, resulting in multiple bruises, petechiae, and hemorrhage into the tissues. Causes include infection and drug sensitivity and toxicity. A diagnosis is reached only by the exclusion of other causes. Considered to be a manifestation of an autoimmune response, two distinct entities, acute and chronic thrombocytopenia, can be differentiated on clinical manifestations alone. The acute form usually occurs in children between 2 and 6 years of age and is benign, with complete recovery usually apparent within 6 weeks. The chronic form usually occurs in adults between 20 and 50 years of age. Recovery is rarely spontaneous and often requires adrenocortical steroids or splenectomy. Compare disseminated intravascular coagulation. See also hemophilia, hemorrhagic diathesis, idiopathic thrombopenic purpura, thrombasthenia.

observations Common manifestations include petechiae and ecchymoses on the skin, particularly the lower extremities; easy bruising; bleeding from the nose and gums; melena in stools; hematemesis; heavy menses and breakthrough bleeding; and hematuria. Jaundice, fever, and decreased levels of consciousness may be seen in thrombotic thrombocytopenic purpura. Diagnosis focuses on obtaining a history of bleeding symptoms and on ruling out other causes of thrombocytopenia, such as medications, ethanol abuse, HIV, or hematologic disorder. Lab findings include decreased platelet count. Bleeding time is prolonged, but coagulation time is normal. Capillary fragility is increased. Bone marrow aspiration shows an abundance of megakaryocytes. In thrombotic thrombocytopenic purpura there is severe anemia, elevated BUN, elevated creatinine, elevated reticulocytes, elevated LDH, decreased haptoglobin, and fragmented RBCs on peripheral smear. Platelet size and morphologic appearance may be abnormal in thrombotic thrombocytopenic purpura. Complications include hemorrhage into organs, such as the brain, gastrointestinal tract, or heart, which can be fatal without treatment.

interventions Medications that may be causing or contributing to the thrombocytopenia are discontinued. Treatment is determined by platelet count and bleeding status. Corticosteroids are used to enhance platelet production and promote capillary integrity. Immunosuppressants are used if the disease does not respond to steroids. Platelet transfusions are used in cases of severe bleeding in idiopathic thrombocytopenic purpura. Plasma exchange or plasmapheresis is the treatment of choice in thrombotic thrombocytopenic purpura. VinCRIStine may be used in thrombotic thrombocytopenic purpura cases that are refractory to plasmapheresis. Splenectomy may be considered for severe unresponsive thrombocytopenia. Immune globulin is given to prepare severely thrombocytic individuals for surgery. Platelet counts and bleeding episodes are monitored closely. Stool softeners are administered to prevent constipation.

nursing considerations Nursing goals are aimed at eliminating gross or occult bleeding, maintaining vascular integrity, decreasing risk for injury, and reducing complications. Safety precautions are instituted to prevent bruising (e.g., mouth swabs and soft bristle toothbrush for oral care; electric razor for shaving; insertion of IV access device for blood draws; padding bed rails and hard surfaces). Emesis, sputum, stool, urine, and other secretions for occult blood and pad counts during menstruation are frequently assessed and tested. Active bleeding is controlled with ice packs, gentle pressure, or packing. Rest and activity should be carefully balanced to conserve energy. Education is necessary about trauma prevention and safety precautions; avoidance of contact sports; avoidance of Valsalva's maneuver; the necessity for gentle coughing, sneezing, and nose blowing; and the necessity for increased fluid intake and balanced periods of rest and exercise. Instruction is given in infection precautions for those taking immunosuppressants. Education includes instruction to avoid anticoagulant over-the-counter medications, such as aspirin/aspirin products and other NSAIDs.

id·i·o·path·ic throm·bo·cy·to·pe·nic pur·pu·ra

(ITP) (id'ē-ō-path'ik throm'bō-sī-tō-pē'nik pŭr'pyŭr-ă)

A systemic illness characterized by extensive ecchymoses and hemorrhages from mucous membranes and very low platelet counts; resulting from destruction in the spleen of platelets to which an autoimmune globulin is bound; childhood cases, which often follow viral infection, are mild and transitory; in adults, bleeding may be recurrent and severe. Synonym(s): immune thrombocytopenic purpura, purpura hemorrhagica, thrombopenic purpura.

purpura

(pur'pyu-ra) [L. purpura, purple]

PURPURA

Any rash in which blood cells leak into the skin or mucous membranes, usually at multiple sites. Purpuric rashes often are associated with disorders of coagulation or thrombosis. Pinpoint purpuric lesions are called petechiae; larger hemorrhages into the skin are called ecchymoses. Synonym: peliosis See: illustration

allergic purpura

Any of a group of purpuras caused by a variety of agents, including bacteria, drugs, and food. The immune complexes associated with type III hypersensitivity reaction damage the walls of small blood vessels, leading to bleeding. Synonym: nonthrombocytopenic purpura

hemorrhagic purpura

Henoch-Schönlein purpura

idiopathic thrombocytopenic purpura

Abbreviation: ITP

A hemorrhagic autoimmune disease in which there is destruction of circulating platelets, caused by antiplatelet autoantibodies that bind with antigens on the platelet membrane, making platelets more susceptible to phagocytosis and destruction in the spleen. It occurs as an acute disease in children, usually between ages 2 and 6, and often follows a viral infection. Chronic ITP seldom follows an infection and is commonly linked to immunologic disorders such as lupus erythematosis or patients with acquired immunodeficiency syndrome who are exposed to the rubella virus. It also is linked to drug reactions, and occurs in cases of alcohol, heroin, or morphine abuse. It mainly affects adults younger than age 50, especially women between 20 and 40. Opsonization of platelets by autoantibodies stimulates their lysis by macrophages, esp. in the spleen. Synonym: Henoch-Schönlein disease; hemorrhagic purpura; thrombocytopenic purpura; thrombopenic purpuraillustration;

CAUTION!

People with ITP should take special precautions to avoid injuries in contact sports. Aspirin and other drugs that may cause bleeding should only be taken by people with ITP under direction of an experienced physician.

Symptoms

Symptoms may include bleeding from the nose, the gums, or the gastrointestinal tract. Physical findings include petechiae, esp. on the lower extremities, and ecchymoses. Laboratory findings: The platelet count is usually less than 20,000/mm3, bleeding time is prolonged, and may be associated with mild anemia as a result of bleeding.

Treatment

If patients are asymptomatic (i.e., have no active bleeding) and have platelet counts of about 50,000/mm3, treatment is not needed (4 out of 5 patients recover without treatment). Treatment for symptomatic patients, or patients with very low platelet counts, usually is with glucocorticoids or immune globulin for acute cases and corticosteroids for chronic cases. For those who do not respond within 1 to 4 months, treatment may include high-dose corticosteroids, intravenous immune globulin (IVIG), immunosupression, immunoabsorption apheresis using staphylococcal protein-A columns to filter antibodies out of the bloodstream, AntiRhD therapy for those with specific blood types, splenectomy, or chemotherapeutic drugs such as vincristine or cyclophosphamide.

Patient care

Platelet count is monitored closely. The patient is observed for bleeding (petechiae, ecchymoses, epistaxis, oral mucous membrane or GI bleeding, hematuria, menorrhagia) and stools, urine, and vomitus are tested for occult blood. The amount of bleeding or size of ecchymoses is measured at least every 24 hr. Any complications of ITP are monitored. The patient is educated about the disorder, prescribed treatments, and importance of reporting bleeding (such as epistaxis, gingival, urinary tract, or uterine or rectal bleeding) and signs of internal bleeding (such as tarry stools or coffee-ground vomitus). The patient should avoid straining during defecation or coughing because both can lead to increased intracranial pressure, possibly causing cerebral hemorrhage. Stool softeners are provided as necessary to prevent tearing of the rectal mucosa and bleeding due to passage of constipated or hard stools. The purpose, procedure, and expected sensations of each diagnostic test are explained. The role of platelets and the way in which the results of platelet counts can help to identify symptoms of abnormal bleeding are also explained. The lower the platelet count falls, the more precautions the patient will need to take; in severe thrombocytopenia, even minor bumps or scrapes can result in bleeding. The nurse guards against bleeding by taking the following precautions to protect the patient from trauma: keeping the side rails of the bed raised and padded, promoting use of a soft toothbrush or sponge-stick (toothette) and an electric razor, and avoiding invasive procedures if possible. When venipuncture is unavoidable, pressure is exerted on the puncture site for at least 20 min or until the bleeding stops. During active bleeding, the patient maintains strict bedrest, with the head of the bed elevated to prevent gravity-related intracranial pressure increases, possibly leading to intracranial bleeding. All areas of petechiae and ecchymoses are protected from further injury. Rest periods are provided between activities if the patient tires easily. Both patient and family are encouraged to discuss their concerns about the disease and its treatment, and emotional support is provided and questions answered honestly. The nurse reassures the patient that areas of petechiae and ecchymoses will heal as the disease resolves. The patient should avoid taking aspirin in any form as well as any other drugs that impair coagulation, including nonsteroidal anti-inflammatory drugs. If the patient experiences frequent nosebleeds, the patient should use a humidifier at night and should moisten the nostrils twice a day with saline. The nurse teaches the patient to monitor the condition by examining the skin for petechiae and ecchymoses and demonstrates the correct method to test stools for occult blood. If the patient is receiving corticosteroid therapy, fluid and electrolyte balance is monitored and the patient is assessed for signs of infection, pathological fractures, and mood changes. If the patient is receiving blood or blood components, they are administered according to protocol; vital signs are monitored before, during, and after the transfusion, and the patient is observed closely for adverse reactions. If the patient is receiving immunosuppressants, the patient is monitored closely for signs of bone marrow depression, opportunistic infections, mucositis, GI tract ulceration, and severe diarrhea or vomiting. If the patient is scheduled for a splenectomy, the nurse determines the patient's understanding of the procedure, corrects misinformation, administers prescribed blood transfusions, explains postoperative care and expected activities and sensations, ensures that a signed informed consent has been obtained, and prepares the patient physically (according to institutional or surgeon's protocol) and emotionally for the surgery. Postoperatively, all general patient care concerns apply. Normally, platelets increase spontaneously after splenectomy, but the patient may need initial postoperative support with blood and component replacement and platelet concentrate. The patient with chronic ITP should wear or carry a medical identification device.

purpura nervosa

An obsolete term for Henoch-Schönlein purpura.

nonthrombocytopenic purpura

posttransfusion purpura

Abbreviation: PTP

An abnormal immune-mediated fall in the number of circulating platelets, caused by a recipient's reaction to foreign antigens on platelets received during a transfusion. Although the immune reaction normally starts against the donated platelets, in PTP host (recipient) platelets are also attacked, leading to a severe decrease in platelet numbers about a week, plus or minus 2 days, after the platelet transfusion. The consequences of a low platelet count may include bleeding, bruising, or discoloration of the skin.

purpura rheumatica

Purpura with joint pain, colic, bloody stools, and vomiting of blood.

senile purpura

Purpura occurring in debilitated and aged people with ecchymoses and petechiae on the legs.

purpura simplex

Purpura that is not associated with systemic illness.

thrombocytopenic purpura

thrombopenic purpura

thrombotic thrombocytopenic purpura

Abbreviation: TTP

A rare, life-threatening disease marked by widespread aggregation of platelets throughout the body, neurological dysfunction, and renal insufficiency. The disease is triggered by a deficiency of an enzyme that cleaves von Willebrand factor (a blood clotting protein). This deficiency results in blood clots in small blood vessels throughout the body. Shifting neurological signs such as aphasia, blindness, and convulsions are often present. See: hemolytic uremic syndrome

Etiology

The disease has occurred in patients taking certain drugs (e.g., ticlopidine); in some patients with cancer or HIV-1 infection; and in some pregnant women.

Treatment

Plasmapheresis or infusions of fresh frozen plasma are effective in treating the disease.

wet purpura

A blister filled with blood; colloquially, a blood blister.

thrombocytopenic purpura

Visible haemorrhages into the skin, mucous membranes and elsewhere resulting from a decreased number of PLATELETS (thrombocytes) per unit volume of blood. Platelets are necessary for normal clotting of the blood.

throm·bo·cy·to·pe·nic pur·pu·ra

(throm'bō-sī'tō-pēn'ik pŭr'pyŭr-ă)

A systemic illness characterized by extensive ecchymoses and hemorrhages from mucous membranes and low platelet counts; resulting from platelet destruction by macrophages due to an antiplatelet factor.

purpura

a hemorrhagic disease characterized by extravasation of blood into the tissues, under the skin and through the mucous membranes, and producing spontaneous ecchymoses and petechiae on the skin. Similar lesions are produced in many specific diseases, e.g. epizootic hemorrhagic disease of deer, bracken poisoning in cattle, and leptospirosis in calves. In immune-mediated purpura there is a defect in the integrity of the vessel wall due to immunological mechanisms, which may also cause a thrombocytopenia.

a well-defined disease of horses, occurring sporadically, usually associated with a respiratory tract infection. Clinical signs include cold, subcutaneous, edematous swellings, usually about the head and not always symmetrical, mucosal petechiation and high heart rate; affected horses commonly die within a few days. Nonthrombocytopenic.

neonatal purpura

alloimmune hemolytic anemia of the newborn in pigs is sometimes accompanied by a thrombocytopenic purpura, caused by antiplatelet antibodies. See also alloimmune thrombocytopenia.

nonthrombocytopenic purpura

purpura without any decrease in the platelet count of the blood. In such cases the cause of purpura is either abnormal capillary fragility or a clotting factor deficiency.

thrombocytopenic purpura

purpura associated with a decrease in the number of platelets in the blood. See also immune-mediated thrombocytopenia.

vascular purpura

that caused by loss of vascular integrity or function, as seen in vitamin C deficiency (scurvy), diabetes mellitus and hyperadrenocorticism.

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